I-thalassemia ye-Beta yi- anemia ezuze ilifa apho umzimba ongakwazi ukuvelisa i-hemoglobin ngokuqhelekileyo. I-Hemoglobin A (i-hemoglobin enkulu kubantu abadala) iqulethe ezimbini ezimbini ze-globin kunye neetoni ezimbini ze-globin. Kwi-beta thalassemia, umongo weethambo awukwazi ukuvelisa inani eliqhelekileyo le-glob bethi ngenxa yokungabi naso ku-hemoglobin A. Kanjalo, kukho i-globin ye-alphab engaphezu kwe-glob ephazamisayo eyaziwayo njenge-hemolysis.
Ngubani Osemngciphekweni?
I-beta thalassemia yimeko ezuze ilifa. Kudinga ukuba bobabini abazali ukuba bathwale isifo esibizwa ngokuba yi-beta thalassemia trait or minor. Xa bobabini abazali bephethe i-beta thalassemia, bane-1 kwi-4 ithuba lokuba nomntwana onesifo se-beta thalassemia. Ngelishwa, kuba uphawu lwe-beta thalassemia alubanganga iimpawu, abazali abanokungazi le mngcipheko ngaphambi kokukhulelwa.
Ziziphi iintlobo zeBeta iThalassemia?
I-thalassemia ye-Beta ingahlelwa ngeendlela ezimbini: ngokuguqulwa kofuzo okanye i-need transfusion.
Utshintsho lwezityalo
I-Beta zero thalassemia : I-zero kwi-beta zero mutation ibonisa ukuba ayikho i-gloa ye-beta inokuveliswa yi-chromosome.
I-Beta kunye ne-thalassemia : i-globita ye-Beta ikhiqizwa ngexabiso elincinci. Ixabiso le-beta globin eveliswayo lihluka kakhulu kunye nokuguqulwa okuthethiweyo. Ezinye izitshintsho zivelisa phantse i-glob i-glob ekwenza i-klinikhi efana ne-beta zero.
Iimfuno zokudluliselwa
I-beta enkulu ye-thalassemia: I- beta enkulu ye-thalassemia ichazwe yidingo lokuxilongwa kwexesha elide, ngokuqhelekileyo ngenyanga (kuthiwa yi-hypertransfusion therapy).
I-Beta thalassemia intermedia : Ngokuqhelekileyo ukumodareyitha i-anemia engadinga ukumpontshelwa ngamanye amaxesha (ngexesha lokugula, ubusuku, njl) kodwa kungekho rhoqo.
I-beta ye-thalassemia / umncinci : Oku kubangela ukuba i-anemia ephazamisekile iqheleke ngokuqhelekileyo kwi-regular count blood count.
I-Beta iThalassemia ichongwa njani?
Ngokubanzi, i-beta thalassemia enkulu ichongiwe kwiskrini esandula . Kule ntsana, uvavanyo luya kubona kuphela i-hemoglobin F (okanye i-fetal), ayikho i-hemoglobin A. La bantwana bathunyelwa kwi-hematologist ukuba bajongwe ngokukhawuleza. Ezinye iziganeko ezichaphazelekayo ezichaphazelekayo kwi-beta thalassemia ziphakathi ziya kuchongwa ngale ndlela.
Ezinye izigulane ezichaphazelekayo ziza kuchongwa kwi- count full blood count (CBC). I-CBC iya kubonakalisa i-anemia elula kunye neyomodareyitha kunye neeseli ezincinci zegazi ezibomvu. Oku kungadideka ngentswelo ye-anemia. Ukuxilongwa kuqinisekiswa yiprofayili ye-hemoglobin (ebizwa ngokuba yi-electrophoresis). Kwi-beta thalassemia intermedia kunye nomxholo lo vavanyo lubonisa ukuphakama kwe-hemoglobin A2 (ifom ye-2 ye-hemoglobin yabantu abadala) ngamanye amaxesha i-F (fetal).
Ziziphi Iingonyango zeBeta iThalassemia?
I-beta ye-thalassemia impawu ayifuni unyango. Abantu abanomdla we-beta thalassemia baya kuba ne-anemia engapheliyo.
Ukutshintshwa kwegazi: Izigulane ezinkulu ze-beta thalassemia zifuna ukumpontshelwa ubomi. Ulwaphulo lwe-Transfusion lusetyenziselwa ukunqanda ukuveliswa kweefom yegazi ebomvu.
I-beta thalassemia intermedia izigulana zingadinga unyango lokuxilongwa ngexesha lokugula kunye nokukhulelwa (ngexesha lokukhula kancinci).
I-iron chelation yonyango: Izigulane ezinkulu nge-beta thalassemia zifumana i-iron engaphezu kwe-red nge-blood cell. Le nsimbi engaphezulu ingabangela umonakalo kwizitho, ingakumbi intliziyo. I-Iron Chelation inceda umzimba ukuba ususe isinyithi. Ezi ziyakunikwa njengemithi yomlomo okanye ukunyuswa phantsi kwesikhumba. Izigulane nge-beta thalassemia intermedia ziyakwazi ukuphuhlisa i-iron ngaphezu kokungabikho kwegazi lokubanjelwa okwesibini ukwenyuka ukutyunjwa kwentsimbi kwisisu esincinci.
I-Splenectomy : I-spleen ingaba yanda kakhulu ngenxa yokuchithwa kweseli ebomvu (hemolysis) kunye nokuveliswa kweeselfom wegazi ebomini kwipeni. Ukuba ukunyuswa kwegazi kufuneka kwandiswe okanye ezinye izibalo zamaseli zegazi zihla ngenxa yokubamba ngepeni, ityha ingadinga ukuba isuswe.
I-Hydroxyurea: I-Hydroxyurea isetyenziselwe ukwandisa i-fetal hemoglobin production. Impumelelo iye yahluka.